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Pediatric Blood & Cancer Jun 2023Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic...
Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.
Topics: Child; Humans; Surface Plasmon Resonance; Pancreatic Neoplasms; Tomography, X-Ray Computed; Carcinoma, Papillary; Pancreas
PubMed: 36215203
DOI: 10.1002/pbc.29975 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Sep 2022To investigate the clinicopathological characteristics of pancreatic lesions in children. The clinicopathological data of pancreatic lesions in children were analyzed...
To investigate the clinicopathological characteristics of pancreatic lesions in children. The clinicopathological data of pancreatic lesions in children were analyzed including 42 cases of pancreatic tumors diagnosed from January 2000 to May 2021 in Guangzhou Women's and Children's Medical Center, Guangzhou, China. Histological and immunohistochemical assessments were performed Related literature was reviewed. The 42 pediatric patients with pancreatic lesions aged 1 day to 12 years (mean, 4.25 years). There were 23 males and 19 females. Clinical presentations included abdominal masses, abdominal pain, vomiting and persistent hypoglycemia after birth. Ultrasound and computerized tomography examination showed space-occupying pancreatic lesions in 31 cases, but no detectable pancreatic lesions in 11 cases. Histologically, among the 42 cases, 22 cases (52.4%) were neoplastic, including 18 cases of epithelial origin. Nine cases of pancreatoblastoma showed that the epithelial tumor cells were arranged in a trabecular pattern, with squamous nests. Six cases of solid-pseudopapillary tumors revealed hemorrhagic and necrotic cysts and monomorphic epithelioid cells arranged in solid sheets, nests or pseudopapillae. Two cases of neuroendocrine tumors showed tumor cells arranged in cords or nests; one case had a mitotic count of about 3/10 high power field, and a Ki-67 index of about 5%, which was consistent with G2 neuroendocrine tumor; the other case showed tumor cells with cytological atypia, brisk mitoses, about 25/10 HPF and a Ki-67 index of about 80%, consistent with small-cell type neuroendocrine carcinoma. The case of acinar cell carcinoma showed high cellularity, tumor cells in solid, cord-like or acinar-like arrangement with little stroma, and monotonous tumor cells with single distinct nucleolus. There were 4 cases of mesenchymal tumors, including 3 cases of Kaposi's hemangioendothelioma and 1 case of inflammatory myofibroblastic tumor. Among the 20 cases (47.6%) of non-neoplastic lesions, there were 11 cases of hyperinsulinism with ATP-sensitive potassium channel abnormality (HAPCA). Severn cases of diffuse type HAPCA in which the islets scattered between the pancreatic acinar tissue, enlarged, and prominent nuclei. Three cases of focal type HAPCA showed pancreatic islet hyperplasia in the form of nested nodules (0.6-1.5 cm). One case of atypical type HAPCA had extensive islet hyperplasia in pancreatic tissue, and scattered proliferation of nest-like nodules was noted. There were also 7 cases of pseudocyst and 2 cases of congenital cyst. Immunohistochemically, pancreatoblastomas were diffusely positive for CKpan, CK8/18, and β-catenin (nuclear staining of squamous nests only). Solid-pseudopapillary tumors expressed CD10, cyclin D1, CD99, vimentin, CD56, and β-catenin (nuclear staining). Neuroendocrine tumors were positive for CK, Syn, NSE, CgA, CD56, and β-catenin (membranous staining). The acinar cell carcinoma was positive for CK8/18, trypsin, and β-catenin (membranous staining). Pancreatic lesions in children have a wide range of histopathological types. HAPCA is the most common lesion of newborns. Pediatric pancreatic tumors are rare and mostly malignant. It is important to recognize them and make correct pathological diagnoses.
Topics: Carcinoma, Acinar Cell; Carcinoma, Squamous Cell; Child; Female; Humans; Hyperplasia; Infant, Newborn; Ki-67 Antigen; Male; Neuroendocrine Tumors; Pancreatic Neoplasms; beta Catenin
PubMed: 36097903
DOI: 10.3760/cma.j.cn112151-20220302-00144 -
Up-regulation of ALK is associated with altered Wnt/beta-catenin pathway in adult pancreatoblastoma.Pathology Feb 2023
Topics: Humans; Adult; Up-Regulation; beta Catenin; Wnt Signaling Pathway; Receptor Protein-Tyrosine Kinases
PubMed: 35794050
DOI: 10.1016/j.pathol.2022.04.005 -
Journal of Indian Association of... 2022Acinar cell carcinoma (ACC) is a rare malignant tumor of the pancreas. A 10-year-old girl presented with a large tumor arising from the pancreatic head. Excision sans...
Acinar cell carcinoma (ACC) is a rare malignant tumor of the pancreas. A 10-year-old girl presented with a large tumor arising from the pancreatic head. Excision sans Whipple's procedure was performed. Histopathology revealed it as ACC. In the context of this case, this rare tumor is being reported to highlight that such tumors arising from the head of the pancreas can be managed successfully without always resorting to a Whipple's procedure.
PubMed: 35733585
DOI: 10.4103/jiaps.JIAPS_29_21 -
Annales de Pathologie Nov 2022
Topics: Humans; Pancreatic Neoplasms
PubMed: 35701283
DOI: 10.1016/j.annpat.2022.04.002 -
Virchows Archiv : An International... Aug 2022Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here...
Pancreatoblastoma (PB) is a rare tumor of the pancreas. In case of metastases, the treatment options are sparse and targeted approaches are not developed. We here evaluate MCL1 amplification as a putative target in PB.Thirteen samples from adult (10/13) and pediatric patients (3/13) were collected. Three of these samples had been previously subjected to whole-exome sequencing (2 cases) or whole-genome sequencing (1 case) within a precision oncology program (NCT/DKTK MASTER), and this analysis had shown copy number gains of MCL1 gene. We established a fluorescence in situ hybridization (FISH) test to assess the copy number alterations of MCL1 gene in 13 formalin-fixed paraffin-embedded PBs, including the 3 cases assessed by genome sequencing. FISH analysis showed the amplification of MCL1 in 2 cases (both were adult PB), one of which was a case with the highest copy number gain at genomic analysis. In both cases, the average gene copy number per cell was ≥ 5.7 and the MCL1/1p12 ratio was ≥ 2.4. Our data support MCL1 as a putative target in PB. Patients with MCL1-amplified PB might benefit from MCL1 inhibition. Sequencing data is useful to screen for amplification; however, the established FISH for MCL1 can help to determine the level and cellular heterogeneity of MCL1 amplification more accurately.
Topics: Adult; Child; Gene Amplification; Humans; In Situ Hybridization, Fluorescence; Myeloid Cell Leukemia Sequence 1 Protein; Pancreatic Neoplasms
PubMed: 35668118
DOI: 10.1007/s00428-022-03349-w -
Cancer Cytopathology Jul 2022The classification and management of solid pancreatic neoplasms has expanded significantly in recent years because of advances in immunohistochemical markers, molecular... (Review)
Review
The classification and management of solid pancreatic neoplasms has expanded significantly in recent years because of advances in immunohistochemical markers, molecular diagnostics, and therapeutics. Solid pancreatic masses are subdivided into 1) ill-defined, scirrhous, and stroma-rich (ductal adenocarcinoma) and 2) well circumscribed, cellular, and stroma-poor (including neuroendocrine neoplasms, acinar cell carcinoma, pancreatoblastoma, and solid-pseudopapillary neoplasm). Definitive diagnosis, particularly of stroma-poor, circumscribed tumors, requires the incorporation of radiologic and cytologic findings, along with the judicious use of (broad, but limited) immunohistochemical panels (pancytokeratin and neuroendocrine [synaptophysin], acinar [trypsin], and solid-pseudopapillary neoplasm [β-catenin] markers), along with unstained slides. Depending on the initial results, immunohistochemical panels should be expanded to include more confirmatory (acinar and neuroendocrine) markers. This ensures that adequate material is available for definitive diagnosis/subclassification and, in some cases, grading, and/or molecular studies, particularly of grade 3 neuroendocrine neoplasms and mixed-lineage tumors. The objective of this review is to expand the understanding of the cytomorphology of solid pancreatic neoplasms using an integrated, multidisciplinary approach in their diagnosis. Knowledge and understanding of recent updates in the classification of solid pancreatic neoplasms ensures that cytopathologists appropriately triage specimens, judiciously use and interpret ancillary studies, and incorporate these results in reporting.
Topics: Biomarkers, Tumor; Carcinoma, Acinar Cell; Humans; Neuroendocrine Tumors; Pancreatic Neoplasms
PubMed: 35594486
DOI: 10.1002/cncy.22597 -
Pathologica Feb 2022Pediatric solid neoplasms are rare and very different from those observed in adults. The majority of them are referred to as embryonal because they arise as a result of... (Review)
Review
New insights in gastrointestinal "pediatric" neoplasms in adult patients: pancreatoblastoma, hepatoblastoma and embryonal sarcoma of the liver. A practical approach by GIPPI-GIPAD Groups.
Pediatric solid neoplasms are rare and very different from those observed in adults. The majority of them are referred to as embryonal because they arise as a result of alterations in the processes of organogenesis or normal growth and are characterized by proliferation of primitive cells, reproducing the corresponding tissue at various stages of embryonic development. This review will focus on embryonal gastrointestinal pediatric neoplasms in adult patients, including pancreatoblastoma, hepatoblastoma, and embryonal sarcoma of the liver. Although they are classically considered pediatric neoplasms, they may (rarely) occur in adult patients. Hepatoblastoma represents the most frequent liver neoplasm in the pediatric population, followed by hepatocellular carcinoma and embryonal sarcoma of the liver; while pancreatoblastoma is the most common malignant pancreatic tumor in childhood. Both in children and adults, the mainstay of treatment is complete surgical resection, either up front or following neoadjuvant chemotherapy. Unresectable and/or metastatic neoplasms may be amenable to complete delayed surgery after neoadjuvant chemotherapy. However, these neoplasms display a more aggressive behavior and overall poorer prognosis in adults than in children, probably because they are diagnosed in later stages of diseases.
Topics: Child; Female; Hepatoblastoma; Humans; Liver Neoplasms; Pancreatic Neoplasms; Pregnancy; Sarcoma
PubMed: 35212317
DOI: 10.32074/1591-951X-559 -
Translational Oncology Apr 2022The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or...
PURPOSE
The aim of this study is to analyze the clinical and pathological features of pancreatoblastoma (PB) and to obtain better management for patients with relapsed or metastatic disease.
METHODS
Four cases treated in our institution and 59 cases reported previously in the literature from the PubMed biomedical database (2000-2020) were reviewed and analyzed.
RESULTS
Four cases with PB presented with abdominal pain and palpable abdominal masses, with the tumor size ranging from 5.2 to 18 cm in diameter. The invasion of the splenic vein and superior mesenteric artery, duodenum, and lymph nodes were risk factors for PB. Three cases were treated with combination therapy and showed favorable outcomes, while one case was treated with chemotherapy alone due to tumor progression and died of the disease. Squamous corpuscles were revealed in the tumor samples and considered a defining component for histological diagnosis.
CONCLUSIONS
Multidisciplinary diagnosis plays an important role in clinical management. The risk factors should be considered in the therapeutic stratification of PB before surgery.
PubMed: 35180620
DOI: 10.1016/j.tranon.2022.101359 -
PancreasPediatric pancreatoblastoma (PBL) is a rare disease, and the treatment of which is diverse. The molecular alteration in pancreatoblastoma is not very clear. A 7-year-old...
Pediatric pancreatoblastoma (PBL) is a rare disease, and the treatment of which is diverse. The molecular alteration in pancreatoblastoma is not very clear. A 7-year-old female who presented with intermittent abdominal pain, anorexia, and abdominal mass was admitted in our hospital. Pancreaticoduodenectomy, cholecystectomy, and retroperitoneal lymph node dissection were conducted. Immunohistochemistry results after surgery showed creatine kinase +, clusters differentiation 56 -, clusters differentiation 99 +, carcinoembryonic antigen -, periodic acid-Schiff +, B- catenin +, Ki-67 + 70%, progesterone receptor +, neuron-specific enolase -, vimentin -, and insulin -. According to the cell shape and the results of immunohistochemistry, the patient was diagnosed with PBL. The tumor tissue, adjacent tissue, and blood were collected. Mutation profiles were detected using next-generation sequencing technique with a panel of 704 genes. The child recovered well without complications postoperatively. There were 261 genes mutated in her plasma or tumor tissue (mutant frequency ≥1%). The adjacent tissue and plasma harbored the echinoderm microtubule-associated protein-like 4 gene-anaplastic lymphoma kinase fusion and tumor tissue harbored proto-oncogene receptor tyrosine kinase 1-solute carrier family 34 member A2 fusion. The gene alteration profiles of PBL patients warrant further investigations, which may provide new insight into the treatment of this disease.
Topics: Abdominal Pain; Anorexia; Child; Female; Genetic Testing; Humans; Pancreatectomy; Pancreatic Neoplasms
PubMed: 35041346
DOI: 10.1097/MPA.0000000000001926